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Carbohydrate-deficient Glycoprotein Syndrome Type I: Disease Bioinformatics

Research of Carbohydrate-deficient Glycoprotein Syndrome Type I has been linked to Congenital Disorders Of Glycosylation, Congenital Disorders, Nervousness, Hypoplasia, Congenital Cerebellar Hypoplasia. The study of Carbohydrate-deficient Glycoprotein Syndrome Type I has been mentioned in research publications which can be found using our bioinformatics tool below. Researched pathways related to Carbohydrate-deficient Glycoprotein Syndrome Type I include Glycosylation, Coagulation, Blood Coagulation, Localization, Protein Glycosylation. These pathways complement our catalog of research reagents for the study of Carbohydrate-deficient Glycoprotein Syndrome Type I including antibodies and ELISA kits against STRABISMUS, IA, ANTITHROMBIN, AHCY, SERPINC1.

Top Research Reagents

We have 1889 products for the study of Carbohydrate-deficient Glycoprotein Syndrome Type I that can be applied to Chromatin Immunoprecipitation, Flow Cytometry, Immunocytochemistry/ Immunofluorescence, Immunohistochemistry, Western Blot from our catalog of antibodies and ELISA kits.

NBP1-31528
Western Blot: GDI1 Antibody [NBP1-31528] - Sample (30 ug of whole cell lysate) A: Hep G2 10% SDS PAGE; antibody diluted at 1:1000.Immunohistochemistry-Paraffin: GDI1 Antibody [NBP1-31528] - Sample: Paraffin-embedded mouse lung. GDI1 antibody dilution: 1:500.

Rabbit Polyclonal
Species Human, Mouse
Applications WB, IHC, IHC-P

NBP1-87055
Western Blot: PMM1/Phosphomannomutase 1 Antibody [NBP1-87055] - Lane 1: NIH-3T3 cell lysate (Mouse embryonic fibroblast cells). Lane 2: NBT-II cell lysate (Rat Wistar bladder tumor cells).Immunohistochemistry-Paraffin: PMM1/Phosphomannomutase 1 Antibody [NBP1-87055] - Staining of human pancreas shows moderate nuclear positivity in exocrine glandular cells.

Rabbit Polyclonal
Species Human, Mouse, Rat
Applications WB, IHC, IHC-P

NBP2-02043
Western Blot: Mannose Phosphate Isomerase Antibody (1C7) [NBP2-02043] - HEK293T cells were transfected with the pCMV6-ENTRY control (Left lane) or pCMV6-ENTRY Mannose Phosphate Isomerase (Right lane) cDNA for 48 hrs and lysed. Equivalent amounts of cell lysates (5 ug per lane) were separated by SDS-PAGE and immunoblotted with anti-Mannose Phosphate Isomerase.Immunocytochemistry/Immunofluorescence: Mannose Phosphate Isomerase Antibody (1C7) [NBP2-02043] Staining of COS7 cells transiently transfected by pCMV6-ENTRY Mannose Phosphate Isomerase.

Mouse Monoclonal
Species Human, Rat
Applications WB, Flow, ICC/IF

NBP2-32539
Immunocytochemistry/Immunofluorescence: ALG1 Antibody [NBP2-32539] - Staining of human cell line Hep G2 shows localization to nucleoli fibrillar center & endoplasmic reticulum.Immunohistochemistry-Paraffin: ALG1 Antibody [NBP2-32539] - Staining of human testis shows strong cytoplasmic positivity in cells in seminiferus ducts, Leydig cells shows moderate staining.

Rabbit Polyclonal
Species Human
Applications ICC/IF, IHC, IHC-P

AF2258
VLDL R was detected in immersion fixed frozen sections of mouse embryo using Mouse VLDL R Antigen Affinity-purified Polyclonal Antibody (Catalog # AF2258) at 15 µg/mL overnight at 4 °C. Tissue was stained using the Anti-Goat HRP-DAB Cell & Tissue Staining Kit (brown; Catalog # <a class=    VLDL R  was detected in perfusion fixed frozen sections of mouse kidney using Goat  Anti-Mouse VLDL R Antigen Affinity-purified Polyclonal Antibody  (Catalog # AF2258) at 3 µg/mL for 1 hour at room  temperature followed by incubation with the Anti-Goat IgG VisUCyte™  HRP Polymer Antibody (Catalog # <a class=

Goat Polyclonal
Species Mouse
Applications WB, IHC

19 Publications
AF482
Ret was detected in perfusion fixed frozen sections of mouse spinal cord using Goat Anti-Mouse Ret Antigen Affinity-purified Polyclonal Antibody (Catalog # AF482) at 15 µg/mL overnight at 4 °C. Tissue was stained using the Anti-Goat HRP-DAB Cell & Tissue Staining Kit (brown; Catalog # <a class=ERBB-family signaling molecules in rat testis cells. (a) Polypeptides in the EGF super-family signal by activating ERBB-family transmembrane receptor tyrosine kinases. ERBB1 is a receptor for ‘classical’ low molecular weight EGF-like peptides. ERBB2 is the primary transducer for ligand-bound ERBB1, ERBB3 and ERBB4. ERBB2’s extracellular domain does not bind known ligands. ERBB3 is a receptor for Neuregulin-1 (NRG1), NRG2 and Neuroglycan-C (CSPG5). Ligand bound ERBB3 displays poor kinase activity and signals most effectively as a heteromer with ERBB1, ERBB2 and/or ERBB4. ERBB4 is a receptor for NRG1, NRG2, NRG3 and NRG4 plus other EGF-like peptides*. (b) Western blotting analysis of ERBB-family proteins in fractions of testis cells from 23-day-old rats. Lysates of type A spermatogonia after proliferating for ~180 days/15 passages in culture (SgL), freshly isolated laminin-binding type A spermatogonia (Sg), laminin non-binding spermatogenic cells (Scy), tubular somatic cells (SC), interstitial somatic cells (IC), MCF7 human mammary gland cells (MCF) and COS7 monkey kidney cells (COS). Arrowheads: ERBBs 1–4 (~185 kDa), RET (~155 and 170 kDa) and TUBA1a (~55 kDa). (c) Relative abundance (qtPCR) of ERBB-family transcripts in testis cells isolated from 23-day-old rats (n=cells from three different rats; ±S.E.M.). Spermatogonia (Sg), Spermatocytes (Scy; differentiating spermatogonia/early spermatocytes), Tubular somatic cells (SC) and Interstitial somatic cells (IC) are cell types described in panel (b). (d) Testis cross-section from 26-day-old tgGCS-EGFP transgenic rats labeled with anti-ERBB2 (Red) overlaying EGFP fluorescence from germ cells (green). Note, cytoplasmic ERBB2 labeling in germ cells resembling differentiating spermatogonia (white arrows) and spermatocytes (yellow arrow). Scale, 40 μm. (e) Rat seminiferous tubule whole mount from 24-day-old wild-type rat labeled using antibodies to ERBB2 (Red) and ZBTB16 (Green). Scale, 20 μm. Note: nuclear ZBTB16 labeling is more robust in ERBB2-dim spermatogonia (cyan arrows), compared with ERBB2-bright spermatogenic cells (white arrows). (f) Rat seminiferous tubule whole mount from a 24-day-old wild-type rat labeled with antibodies to ERBB2 (Red) and phospho-Histone-3 (pH3, Green). Scale, 40 μm. Note: nuclear pH3 in large mitotic ERBB2+ syncytia. Image collected and cropped by CiteAb from the following open publication (https://www.nature.com/articles/cddiscovery201518), licensed under a CC-BY license. Not internally tested by R&D Systems.

Goat Polyclonal
Species Mouse
Applications WB, IHC

40 Publications
AF1267

Goat Polyclonal
Species Human
Applications WB, IP, Neut

6 Publications
AF1060
Western blot shows lysates of mouse adipose tissue and mouse colon tissue. PVDF membrane was probed with 0.5 µg/mL of Goat Anti-Mouse Decorin Antigen Affinity-purified Polyclonal Antibody (Catalog # AF1060) followed by HRP-conjugated Anti-Goat IgG Secondary Antibody (Catalog # <a class=        Simple  Western lane view shows lysates of mouse adipose tissue and mouse colon  tissue, loaded at 0.2 mg/mL. A specific band was detected for  Decorin at approximately 94 kDa (as indicated) using  10 µg/mL of Goat Anti-Mouse Decorin Antigen Affinity-purified  Polyclonal Antibody (Catalog # AF1060) followed by 1:50 dilution of  HRP-conjugated Anti-Goat IgG Secondary Antibody (Catalog # <a class=

Goat Polyclonal
Species Mouse
Applications WB, Simple Western, IHC

     1 Review

24 Publications
AF2747
Clusterin was detected in perfusion fixed frozen sections of mouse liver using Goat Anti-Mouse Clusterin Antigen Affinity-purified Polyclonal Antibody (Catalog # AF2747) at 15 µg/mL overnight at 4 °C. Tissue was stained using the Anti-Goat HRP-DAB Cell & Tissue Staining Kit (brown; Catalog # <a class=Clusterin was detected in perfusion fixed frozen sections of mouse intestine using Goat Anti-Mouse Clusterin Antigen Affinity-purified Polyclonal Antibody (Catalog # AF2747) at 15 µg/mL overnight at 4 °C. Tissue was stained using the Anti-Goat HRP-DAB Cell & Tissue Staining Kit (brown; Catalog # <a class=

Goat Polyclonal
Species Mouse
Applications WB, IHC, IP

13 Publications
NBP2-37322
Western Blot: Serpin A7/TBG Antibody (5B11E9) [NBP2-37322] - Western blot analysis using SERPINA7 mAb against HEK293 (1) and SERPINA7 (AA: 168-302)-hIgGFc transfected HEK293 (2) cell lysate.Immunocytochemistry/Immunofluorescence: Serpin A7/TBG Antibody (5B11E9) [NBP2-37322] - Immunofluorescence analysis of A431 cells using SERPINA7 mouse mAb (green). Blue: DRAQ5 fluorescent DNA dye.

Mouse Monoclonal
Species Human
Applications WB, ELISA, Flow

2914-HT


Species Human
Applications BA

51 Publications
NBP2-48817
Western Blot: Adenosylhomocysteinase/AHCY Antibody [NBP2-48817] -  Analysis in human cell line CACO-2 and human cell line HeLa.Immunocytochemistry/Immunofluorescence: Adenosylhomocysteinase/AHCY Antibody [NBP2-48817] - Staining of human cell line CACO-2 shows localization to cytosol. Antibody staining is shown in green.

Rabbit Polyclonal
Species Human
Applications WB, ICC/IF, IHC

NBP2-57753
PMM2/Phosphomannomutase 2 Antibody [NBP2-57753] - Western blot -Analysis using Anti-PMM2 antibody NBP2-57753 (A) shows similar pattern to independent antibody (B).Immunocytochemistry/Immunofluorescence: PMM2/Phosphomannomutase 2 Antibody [NBP2-57753] - Staining of human cell line SH-SY5Y shows localization to nucleus & cytosol.

Rabbit Polyclonal
Species Human
Applications WB, ICC/IF, IHC

NBP2-60655
Western Blot: ERG Antibody [NBP2-60655] - Total protein from Human THP-1, Jurkat and K562 cells was separated on a 7.5% gel by SDS-PAGE, transferred to PVDF membrane and blocked in 5% non-fat milk in TBST. The membrane was probed with 2.0 ug/ml anti-ERG in blocking buffer and detected with an anti-rabbit HRP secondary antibody using chemiluminescence.Immunocytochemistry/Immunofluorescence: ERG Antibody [NBP2-60655] - HeLa cells were fixed for 10 minutes using 10% formalin and then permeabilized for 5 minutes using 1X TBS + 0.5% Triton-X100. The cells were incubated with anti-ERG at 5 ug/ml overnight at 4C and detected with an anti-rabbit Dylight 488 (Green) at a 1:500 dilution. Alpha tubulin (DM1A) NB100-690 was used as a co-stain at a 1:1000 dilution and detected with an anti-mouse Dylight 550 (Red) at a 1:500 dilution. Nuclei were counterstained with DAPI (Blue). Cells were imaged using a 40X objective.

Rabbit Polyclonal
Species Human
Applications WB, ICC/IF, IHC

NB100-1965
Western Blot: Calnexin Antibody [NB100-1965] - WB analysis of CANX in cell lysates as noted.Western Blot: Calnexin Antibody [NB100-1965] - Subcellular localization of TM6SF2.Immunoaffinity isolation of ER and Golgi complex from mouse liver. ER and Golgi fractions were prepared from mouse liver microsomes by immunoaffinity chromatography as described under Experimental Procedures. Microsome membranes were dissolved in RIPA buffer, and equal volumes were separated on 10% SDS-PAGE and immunoblotting as described under Experimental Procedures. BiP, binding immunoglobulin protein; Gos28, Golgi SNAP receptor complex member 1; *, nonspecific band. Image collected and cropped by Citeab from the following publication (Inactivation of Tm6sf2, a Gene Defective in Fatty Liver Disease, Impairs Lipidation but Not Secretion of Very Low Density Lipoproteins. <i>J Biol Chem</i> (2016) licensed under a CC-BY license.

Rabbit Polyclonal
Species Human, Mouse, Rat
Applications WB, Simple Western, Flow

     3 Reviews

46 Publications
NBP3-03109
Western Blot: Kv11.1 Antibody [NBP3-03109] - Analysis of extracts of various cell lines, using Kv11.1 antibody at 1:500 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL EnhanImmunohistochemistry-Paraffin: Kv11.1 Antibody [NBP3-03109] -  Human lung cancer using KCNH2 antibody at dilution of 1:100 (40x lens).Perform microwave antigen retrieval with 10 mM PBS buffer pH 7.2 before commencing with IHC staining protocol.

Rabbit Polyclonal
Species Human, Mouse
Applications WB, IHC, IHC-P

H00005555-P01
SDS-Page: Recombinant Human PRH2 Protein [H00005555-P01] - 12.5% SDS-PAGE Stained with Coomassie Blue.


Species Human
Applications WB, ELISA, PA


Related Genes

Carbohydrate-deficient Glycoprotein Syndrome Type I has been researched against:

Related PTMs

Carbohydrate-deficient Glycoprotein Syndrome Type I has been studied in relation to posttranslational modifications (PTMs) including:

Alternate Names

Carbohydrate-deficient Glycoprotein Syndrome Type I is also known as Congenital Disorder Of Glycosylation Type 1a, Congenital Disorder Of Glycosylation, Type Ia, Jaeken Syndrome, Phosphomannomutase 2 Deficiency, Carbohydrate-deficient Glycoprotein Syndrome Type 1a.